【光过敏和低补体血症】

伴Evans综合征的狼疮患者发生光过敏和低补体血症更常见

作者：Zhang L 翻译：于若寒（ruohanxue101@126.com） 审校：刘佩玲
发布者：闫冰 审校者：张舸

摘要
本研究目的是探讨SLE伴发Evans综合征的临床特征。本研究为回顾性病例对照研究，比较伴发和不伴发Evans综合征的SLE患者的临床和实验室特点。伴发和不伴发Evans综合征的患者数量以1:3匹配，两组患者年龄和性别均匹配。在5724例住院SLE患者中，我们确定了27例伴发Evans综合征的SLE患者（0.47%，22例女性，5例男性，平均年龄为34.2岁）。15例(55.6%)患者初始表现为血液系统异常，包括6例 (22.2%)孤立的特发性血小板减少性紫癜（ITP），4 例(14.8%)孤立的自身免疫性溶血性贫血（AIHA），5例 (18.5%)经典的Evans综合征。血液系统症状和确诊SLE的平均间隔期为36月（0-252月）。4例(14.8%)患者在确诊SLE后出现Evans综合征，8例(29.6%)患者与SLE同时诊断。SLE并发Evans综合征的患者常出现系统受累，包括发热(55.6%)、浆膜炎(51.9%)、脱发(40.7%)、狼疮肾炎(37%)、雷诺现象(33.3%)、神经精神症状(33.3%)和肺脏受累(25.9%)及光敏(25.9%)。光敏、低补体血症、高血清IgG水平和狼疮肾炎在伴发和不伴发Evans综合征患者中的比例分别是25.9% vs 6.2% (P = 0.007)、88.9% vs 67.1% (P = 0.029)、48.1% vs 24.4% (P = 0.021)和37% vs 64.2% (P = 0.013)。25例(92.6%)患者在接受糖皮质激素、免疫抑制剂和切脾治疗后达到缓解，然而随访中有6例复发，1例死于肾功能衰竭。Evans综合征是SLE罕见的并发症。光敏、低补体血症、高血清IgG水平在Evans综合征患者中较常见，而狼疮肾炎相对少见。一半以上的患者在发病初期就有血液系统表现，经过数月或数年进展为经典的SLE。因此，监测抗体谱和非血液系统表现对ITP或AIHA患者很有必要。

附原文： Abstract：The aim of the study was to investigate the clinical features of systemic lupus erythematous (SLE) complicated with Evans syndrome (ES).We conducted a retrospective case-control study to compare the clinical and laboratory features of age- and gender-matched lupus patients with and without ES in 1:3 ratios.In 5724 hospitalized SLE patients, we identified 27 (0.47%, 22 women and 5 men, average age 34.2 years) SLE patients complicated with ES. Fifteen patients (55.6%) presented with hematologic abnormalities initially, including 6 (22.2%) cases of isolated ITP, 4 (14.8%) cases of isolated AIHA, and 5 (18.5%) cases of classical ES. The median intervals between hematological presentations the diagnosis of SLE was 36 months (range 0-252). ES developed after the SLE diagnosis in 4 patients (14.8%), and concomitantly with SLE diagnosis in 8 patients (29.6%). Systemic involvements are frequently observed in SLE patients with ES, including fever (55.6%), serositis (51.9%), hair loss (40.7%), lupus nephritis (37%), Raynaud phenomenon (33.3%), neuropsychiatric (33.3%) and pulmonary involvement (25.9%), and photosensitivity (25.9%). The incidence of photosensitivity, hypocomplementemia, elevated serum IgG level, and lupus nephritis in patients with ES or without ES was 25.9% vs 6.2% (P = 0.007), 88.9% vs 67.1% (P = 0.029), 48.1% vs 24.4% (P = 0.021), and 37% vs 64.2% (P = 0.013), respectively. Twenty-five (92.6%) patients achieved improvement following treatment of glucocorticoids and immunosuppressants as well as splenectomy, whereas 6 patients experienced the relapse and 1 patient died from renal failure during the follow-up.ES is a relatively rare complication of SLE. Photosensitivity, hypocomplementemia, and elevated serum IgG level were frequently observed in ES patients, but lupus nephritis was less observed. More than half of patients presented with hematological manifestation at onset, and progress to typical lupus over months to years. Therefore, monitoring with antoantibodies profile as well as nonhematological presentations are necessary for patients with ITP and (or) AIHA.

引自：Zhang L, Wu X, Wang L, Li J, Chen H, Zhao Y, Zheng W: Clinical Features of Systemic Lupus Erythematosus Patients Complicated With Evans Syndrome: A Case-Control, Single Center Study. Medicine 2016;95:e3279.